KAWASAKI DISEASE

By Gary M. White, MD

Kawasaki Disease lips Courtesy O. Dale Collins, MD


Kawasaki disease (KD) is a small to medium vessel vasculitis that affects children 6 months to 5 years of age. It is characterized by fever, bright red or chapped lips, strawberry tongue, and a rash. If untreated, a significant portion of patients develop coronary artery disease, e.g., aneurysms.

Etiology

Cases follow wind currents from one Asian country to another. Could Kawasaki disease be precipitated by some environmental wind-blown agent?

Clinical

Kawasaki disease often begins with a high and persistent fever greater than 102 °F, often as high as 104 °F. A persistent fever lasting at least 5 days is considered a classic sign. The fever may last for up to 2 weeks and does not usually go away with normal doses of acetaminophen or ibuprofen.

Types of Skin Rashes

Skin rashes in KD are typically polymorphous, morbilliform, or urticarial. A diaper dermatitis is common. Micropustular and psoriasiform rashes may occur as well. The vasculitis associated with KD may precipitate livedo reticularis, purpura, necrosis, and purpura fulminans.

Kawasaki Disease vs. Drug Reaction

Treatment

Treatment is critical. If untreated, a significant portion of patients develop coronary artery disease, e.g., aneurysms. A specialist in KD should oversee treatment.

The patient is usually hospitalized. An echo to assess cardiac vessels is usually performed. IVIG and high-dose aspirin are given.

The use of infliximab in combination with IVIG reduced hospitalization days and coronary artery inflammation faster than IVIG alone in one study.

Additional Pictures

All photos courtesy O. Dale Collins, MD
Kawasaki Disease conjunctivitis Kawasaki Disease lips

Note the cherry red lips, swollen hands and the rash in the groin.
Kawasaki Disease groin

References

Necrotic vasculitis/purpura fulminans from KD. Dermatol Argent 2009; 15(6):411-419

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